Diagnostic Value of Biological Parameters in Biopsy-Confirmed Thrombotic Microangiopathy-MATRIX Consortium Group.
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Halimi Jean-Michel, Duval Anna, Chardon Etienne, Mesnard Laurent, Frimat Marie, Fakhouri Fadi, Grangé Steven, Servais Aude, Cartery Claire, Coppo Paul, Titeca-Beauport Dimitri, Roger Sébastien, Baroukh Nadine, Fage Nicolas, Delmas Yahsou, Quérard Anne-Hélène, Seret Guillaume, Bobot Mickaël, Le Quintrec Moglie, Ville Simon, von Tokarski Florent, Chauvet Sophie, Wynckel Alain, Martins Manon, Schurder Juliet, Barbet Christelle, Sautenet Bénédicte, Gatault Philippe, Caillard Sophie, Antunes Charles, Bayer Guillaume, Philipponnet Carole, Audard Vincent, Maillard Nicolas, Vuiblet Vincent, Gnemmi Viviane, El Ouafi Zhour, Canet Sébastien, Dekeyser Manon, Piver Éric, Maisons Valentin, MATRIX Consortium Group
Аннотация
METHODS: The presence of common biological parameters was assessed in 967 patients with TMA from 2009 to 2023 (ClinicalTrials.gov: NCT05991245).
RESULTS: The median age was 49 (36-64) years and 53.2% were male. All TMA causes were represented (atypical hemolytic uremic syndrome [aHUS]: 41.6%, drugs: 24.9%, malignancy: 21.4%, autoimmune disease: 18.8%, infection: 7.6%, complement-mediated HUS: 6.8%, organ transplantation: 5.8%, pregnancy: 3.8%, bone marrow transplantation [BMT]: 2.9%, Shiga toxin Escherichia coli hemolytic uremic syndrome [STEC-HUS]: 0.6%, and thrombotic thrombocytopenic purpura [TTP]: 0.6%). The presence of TMA-related parameters concerned virtually all patients with TTP but varied widely for the other patients as follows: anemia: 81.7%, high lactate dehydrogenase (LDH) (75.4%), low haptoglobin (53.7%), and thrombocytopenia (40.3%). Their diagnostic performance was accurate only for TTP. Eleven distinct ways were used for schistocyte metrics and reporting. Relying on schistocyte presence as the single diagnostic criterion would lead to missed diagnosis in 23.8% (STEC-HUS) to 86.4% (BMT) of patients (for anemia: 8.2%-22.3%; thrombocytopenia: 31.8%-67.9%; high LDH: 10.0%-40.7%, low haptoglobin: 0%-70.4%, according to the causes of TMA). The overall risk of missed diagnosis using these parameters was ≥ 50% in all TMA, except in TTP. The best diagnostic performances were obtained when fibrinogen levels were < 5 g/l, creatinine ≥ 300 μmol/l, prothrombin time (PT) < 90%; and when TMA causes were TTP, STEC-HUS, infection, or complement-mediated HUS.
CONCLUSION: Common biological parameters miss the diagnosis in more than 50% of TMA except when fibrinogen is < 5 g/l, creatinine ≥ 300 μmol/l, and PT < 90%. Schistocyte reporting is heterogenous, and its results are usually deceptive in TMA.